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    Researcher Gives an Encouraging Update on Sickle Cell Disease


    "Now that we know that patients are living longer, we need to prepare them for family planning, vocations, and life," Reid says. "That's not something we were able to look at 25 years ago. [Then] we didn't have cause for optimism. Today, we need to encourage kids to be ambitious and prepare for college -- they can do anything they want to do with this disease.

    "Parents need to keep up to date on the latest advances, research, and services so that they can be advocates for their children," Reid says. "They need to work to become part of the health care team along with the nurses and doctors so that if emergencies arise, [they'll] be ready."

    Other major studies have looked at preventing stroke, a devastating event that can lead to irreversible brain damage. One study, according to Reid, showed that blood transfusions can prevent stroke in kids at risk. "As important as this advance is, it requires that we confront issues involving blood transfusion, which in turn carry unique problems," Reid says, citing hepatitis and iron overload. Studies in stem cell transplantation, gene therapy, and the use of cord blood are also underway.

    "This is exciting news," Doris L. Wethers, MD, of the pediatric department at St. Luke's-Roosevelt Comprehensive Sickle Cell Program in New York City, tells WebMD. "There has been a general escalation in emerging [studies] that has resulted in real reductions in the [prevalence of the disease] and in mortality. Our hope is that future studies will continue to show favorable results."

    "These cutting-edge [advances] are encouraging, and it's reassuring to know that the results of trials conducted with adults are transferring to children," Phyllis Harris, MD, tells WebMD. Harris is with the department of pediatrics at George Washington University in Washington.

    Most recent advances in sickle cell disease are moving toward developing more effective therapies that are less toxic to children, Reid says. Use of hydroxyurea, although very helpful in treating sickle cell disease, is somewhat limited due to its side effect of suppressing the bone marrow.

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